{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T06:01:14Z","timestamp":1785564074853,"version":"3.56.0"},"reference-count":10,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T00:00:00Z","timestamp":1785456000000},"content-version":"vor","delay-in-days":211,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Pulmonology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Autoimmune pulmonary alveolar proteinosis (APAP) is caused by impaired surfactant clearance due to neutralizing autoantibodies against granulocyte\u2010macrophage colony\u2010stimulating factor. Although whole\u2010lung lavage and inhaled granulocyte\u2010macrophage colony\u2010stimulating factor therapy are established treatment options, pulmonary fibrosis is increasingly recognized as a clinically relevant complication in a subset of patients with APAP. However, the clinical behavior of APAP\u2010associated fibrosing lung disease and the role of antifibrotic therapy remain unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      A 54\u2010year\u2010old man with a 15\u2010year history of APAP was referred to our institution. High\u2010resolution computed tomography images obtained before referral showed slow progression of reticulation and traction bronchiectasis, suggesting fibrotic progression rather than recurrence of APAP. At presentation, forced vital capacity (FVC) was 3.31\u2009L (80.0% predicted), and diffusion capacity for carbon monoxide (DL\n                      <jats:sub>CO<\/jats:sub>\n                      ) was preserved. During 6 months of observation, FVC declined to 3.03\u2009L (73.5% predicted), accompanied by worsening dry cough and exertional dyspnea. Nintedanib was initiated for a progressive fibrosing phenotype in the context of APAP. Thereafter, FVC remained relatively stable for 2 years, whereas DL\n                      <jats:sub>CO<\/jats:sub>\n                      declined during follow\u2010up.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>APAP\u2010associated fibrosing lung disease may present with a progressive fibrosing phenotype, but its diagnosis and management remain challenging. This case highlights the importance of distinguishing fibrotic progression from recurrence of intra\u2010alveolar proteinosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1155\/crpu\/8073947","type":"journal-article","created":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T05:28:06Z","timestamp":1785562086000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Progressive Fibrosing Lung Disease Treated With Nintedanib in a Patient With Long\u2010Standing Autoimmune Pulmonary Alveolar Proteinosis: A Case Report"],"prefix":"10.1155","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2274-4599","authenticated-orcid":false,"given":"Yoshiaki","family":"Zaizen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuhiko","family":"Nikaido","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeo","family":"Jimi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsuya","family":"Kawano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katsuyuki","family":"Ichiki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Tsuda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0459-4913","authenticated-orcid":false,"given":"Tomoaki","family":"Hoshino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,31]]},"reference":[{"key":"e_1_2_13_1_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202112-2742SO"},{"key":"e_1_2_13_2_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202202-0399ST"},{"key":"e_1_2_13_3_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908681"},{"key":"e_1_2_13_4_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2503643"},{"key":"e_1_2_13_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/rcr2.255"},{"key":"e_1_2_13_6_2","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.01054"},{"key":"e_1_2_13_7_2","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00314-2024"},{"key":"e_1_2_13_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2025-266299"},{"key":"e_1_2_13_9_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200708-1271OC"},{"key":"e_1_2_13_10_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-015-0085-0"}],"container-title":["Case Reports in Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crpu\/8073947","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/crpu\/8073947","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/crpu\/8073947","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T05:28:11Z","timestamp":1785562091000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/crpu\/8073947"}},"subtitle":[],"editor":[{"given":"Venkatkiran","family":"Kanchustambham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":10,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1155\/crpu\/8073947"],"URL":"https:\/\/doi.org\/10.1155\/crpu\/8073947","archive":["Portico"],"relation":{},"ISSN":["2090-6846","2090-6854"],"issn-type":[{"value":"2090-6846","type":"print"},{"value":"2090-6854","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2026-06-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-27","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-31","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"8073947"}}